Salazar Miguel Fdo, Tena Suck Martha Lilia, Rembao Bojórquez Daniel, Salinas Lara Citlaltepetl
Pathology Unit, Neuropathology Service, Mexico General Hospital, Cuauhtémoc, 06726 Mexico City, DF, Mexico.
Department of Neuropathology, National Institute of Neurology & Neurosurgery "Manuel Velasco Suárez", Tlalpan, 14269 Mexico City, DF, Mexico.
Case Rep Pathol. 2016;2016:2494175. doi: 10.1155/2016/2494175. Epub 2016 Jun 30.
Neurilemmomas are benign neoplasms presumedly derived from Schwann cells which rarely originate within the central nervous system. Moreover, their intraventricular location has been seldom noticed with less than 30 cases reported worldwide. Here, we add another case study to the record as well as the fifth one in Latin American population. A 16-year-old boy without significant past clinical data debuted with headache and progressive left eye blindness during six months. Neuroimaging scans showed a bulky, multiloculated, intraventricular tumour emerging from the posterior horn of the left lateral ventricle. Microscopically, the lesion put on view the classical schwannian histology: spindle cells arranged in both compact and loosely textured areas. Verocay bodies were not present but vessel hyalinisation, pericellular reticulin, and senescent atypia were observed. The immunoperoxidase reactions were also consistent with neurilemmal differentiation; however, glial fibrillary acidic protein expression was widespread and unexpectedly seen. Traditionally conceived as "nerve sheath tumours" the dual immunophenotype herein demonstrated points to a different histogenetical pathway other than sheer Schwann cell derivation. As previously advised by some authors, neoplastic transformation from a multipotent stem cell may explain the occasional finding of these tumours in unconventional intracranial compartments.
神经鞘瘤是一种良性肿瘤,推测起源于施万细胞,很少发生于中枢神经系统内。此外,其位于脑室内的情况很少被注意到,全球报道的病例不到30例。在此,我们补充了另一例病例研究,也是拉丁美洲人群中的第五例。一名16岁男孩,既往无重要临床病史,在6个月内出现头痛并逐渐发展为左眼失明。神经影像学扫描显示,一个巨大的、多房性的脑室内肿瘤,起源于左侧脑室后角。显微镜下,病变呈现出典型的施万细胞组织学特征:梭形细胞排列成紧密和疏松纹理区域。未见Verocay小体,但观察到血管玻璃样变、细胞周围网状纤维和衰老异型性。免疫过氧化物酶反应也与神经鞘瘤分化一致;然而,胶质纤维酸性蛋白表达广泛且出乎意料地出现。传统上认为是“神经鞘肿瘤”,本文所展示的双重免疫表型表明其组织发生途径不同于单纯的施万细胞来源。正如一些作者之前所建议的,多能干细胞的肿瘤转化可能解释了这些肿瘤在非常规颅内部位的偶尔发现。